ISSN 2075-3594 (Print)
ISSN 2414-9322 (Online)

Eosinophilic granulomatosis with polyangiitis: оverlap syndrome in eosinophilic diseases

Volgina G.V., Frolova N.F., Mutovina Z.Yu., Iskhakov R.T., Kruglova Т.S., Usatyuk S.S., Pavlov D.P., Fomina D.S.

1) Federal State Budgetary Educational Institution of Higher Education “Russian University of Medicine”, Moscow, Russia; 2) Federal State Budgetary Institution “Moscow Clinical Research Center, Hospital No. 52”, Moscow, Russia; 3) Federal State Budgetary Institution of Continuing Professional Education “Central State Medical Academy of the Administrative Department of the President of the Russian Federation”, Moscow, Russia; 4) Federal State Autonomous Educational Institution of Higher Education “I.M. Sechenov First Moscow State Medical University”, Moscow, Russia
Antineutrophil cytoplasmic antibody-associated vasculitides (ANCA, AAV) are a group of rare necrotizing vasculitides comprising three main entities: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Despite belonging to a common group, EGPA has significant pathophysiological characteristics that affect approaches to its treatment and prognosis. The current definition of EGPA, according to the revision of the vasculitides nomenclature at the Chapel Hill Consensus Conference (2012), includes granulomatous inflammation with abundant eosinophilic infiltration and necrotizing vasculitis of small and medium-sized vessels in a patient with asthma and eosinophilia. This review presents current knowledge on eosinophilic and ANCA-mediated aspects of EGPA pathogenesis, clinical manifestations, diagnostic criteria and approaches to therapy.

Keywords

Eosinophilic granulomatosis with polyangiitis
Churg-Strauss syndrome
eosinophils
hypereosinophilic syndrome
ANCA-associated vasculitis
EGPA classification

About the Authors

Galina V.Volgina – Dr.Sci. (Med.), Professor, Department of Nephrology, Russian University of Medicine. Address: Bldg. 4, 1A Kuskovskaya St., Moscow, 111398; e-mail: volginagv@mail.ru. ORCID: 0000-0002-7771-4074
Nadiya F. Frolova – Cand.Sci. (Med.), Deputy Chief Physician for Nephrology Care, Head of the Interdistrict Nephrology Center, Moscow Clinical Research Center, Hospital No. 52, Moscow Healthcare Department. Address: 3 Pekhotnaya St., Moscow 123182; Associate Professor, Department of Nephrology, Russian University of Medicine, Russian Federation. Address: Bldg. 4, 1A Kuskovskaya St., Moscow, 111398; e-mail: nadiya.frolova@yandex.ru. ORCID: 0000-0002-6086-5220
Zinaida Yu. Mutovina – Cand.Sci. (Med.), Deputy Chief Physician for Medical Affairs, Moscow Clinical Research Center, Hospital No. 52, Moscow Healthcare Department. Address: 3 Pekhotnaya St., Moscow 123182; Associate Professor, Department of Therapy, Cardiology, and Functional Diagnostics, Central State Medical Academy of the Administrative Directorate of the President of the Russian Federation, with a course in Nephrology. Address: Bldg. 1A, 19 Marshal Timoshenko St., Moscow, 121359; e-mail: zmutovina@mail.ru. ORCID: 0000-0001-5809-6015
Daria S. Fomina – Dr.Sci. (Med.), Professor, Deputy Director for Research and Clinical Work, Moscow Clinical Research Center, Hospital No. 52, Moscow Healthcare Department. Address: 3 Pekhotnaya St., Moscow 123182, Chief Allergist-Immunologist, Moscow Healthcare Department, Professor, Department of Clinical Immunology and Allergology, Sechenov First Moscow State Medical University. Address: Bldg. 2, 8 Trubetskaya St., Moscow, 119048; e-mail: daria_fomina@mail.ru. ORCID: 0000-0002-5083-6637
Rustam T. Iskhakov – Head of the Nephrology Intensive Care Unit, Moscow Clinical Research Center, Hospital No. 52, Moscow Healthcare Department. Address: 3 Pekhotnaya St., Moscow 123182; e-mail: stamius@yandex.ru. ORCID: 0000-0003-2850-4465
Sergey S. Usatyuk – Head of Nephrology Department No. 2, Moscow Clinical Research Center, Hospital No. 52, Moscow Healthcare Department. Address: 3 Pekhotnaya St., Moscow 123182; e-mail: usatuk-doc@mail.ru. ORCID: 0000-0002-8742-3860

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